(447) Pemphigus Vegetans for breakfast, lunch and dinner: An unusual clinical presentation of an uncommon condition

Track
Case Reports
Abstract

We present a case of a 21-year-old female who presented with 3 weeks of uncomfortable growths on the axillary and abdominal skin and a small, foul-smelling erosion on the scalp.  She also complained of dry lips.  She reported that she noticed the first lesion after cutting herself shaving. She had bug bites on the legs 3 weeks prior but those resolved. She denied and fevers or fatigue and had a negative COVID-19 test.  On physical exam she had 7 large juicy moist erythematous papules arranged in a linear fashion on the left axilla, as well as 4 lesions of similar morphology in a linear fashion crossing the abdomen, with some crusting. 

A shave biopsy of one of the papules in the axillary vault revealed acanthosis, with papillary dermal edema and suprabasilar acantholysis with abundant eosinophils. DIF was positive for IgG and C3 intracellularly leading to a diagnosis of pemphigus vegetans (Hallopeau type).

Pemphigus vegetans is a localized form of pemphigus vulgaris, with characteristic vegetating plaques at the site of cutaneous erosions, which are commonly found on the scalp, face, and intertriginous areas. It is a relatively rare and benign variant of pemphigus, accounting for only 1-2% of cases. The Hallopeau variant presents with pustular lesions from the outset that are not preceded by bullae and favor flexural areas, whereas the Neumann variant presents with erosions that heal with vegetative plaques and last for longer periods of time.  Pathogenesis is via IgG autoantibodies against intercellular desmoglein leading to acantholysis. We present

Published in: ASDP 61st Annual Meeting

Publisher: The American Society of Dermatopathology
Date of Conference: November 4-10, 2024